Pulmonary hypertension associated with congenital heart disease (PAH-CHD) is one of the most common causes of pulmonary hypertension in children. Patients fall into different subgroups, each requiring specific treatment approaches.
According to an article recently published in the Expert Review of Respiratory Medicine current management is clear for two groups:
- Low pulmonary vascular resistance (PVR) patients: Heart defect should be surgically closed
- Eisenmenger syndrome patients: Heart defect stays open and patients receive pulmonary vasodilator medications
The challenge
The management of patients with open shunts and moderately elevated pulmonary vascular resistance remains challenging. It’s unclear whether surgery to close their heart defect will help or harm them. Currently, doctors use invasive heart catheterization to decide if surgery is safe, but this method has significant limitations.
What’s needed:
- Better non-invasive tests to predict who can safely undergo surgery
- Prospective studies in patients who are diagnosed late
- New therapies like sotatercept and novel biomarkers that could help expand treatment options and redefine which patients can safely have their heart defects repaired
Bottom line: While treatment is straightforward at the extremes, managing the “in-between” patients remains one of the biggest challenges in pediatric pulmonary hypertension.
Read more at this link on the Expert Review of Respiratory Medicine
Citation
Wacker J, Joye R, Beghetti M. Treatment options for children with pulmonary arterial hypertension associated with congenital heart disease. Expert Rev Respir Med. 2025 Oct 30:1-9. doi: 10.1080/17476348.2025.2581340. Epub ahead of print. PMID: 41158018.